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MOD > Immune Mediated Diseases > Flashcards

Immune Mediated Diseases Flashcards

1
Q

Tipoffs for vasculitis

A
  • mononeutritis multiplex
  • palpable purpura
  • “can’t tell why they are sick”
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2
Q

Polyarteritis Nodosa

A

necrotizing vasculitis

  • Etiologies: hep b/c, cryoglobulins
  • Patchy inflammation of all 3 layers of large arteries – true aneurysms (undergo thrombosis)
  • gangrene from ischemia
  • ANCA-negative
  • fibrinoid deposits w/ elastic damage
  • negative hematuria
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3
Q

Microscopic Polyangiitis

A

Positive ANCA, most likely involving capillaries

- hematuria

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4
Q

ANCA responsible for microscopic polyangiitis

A

p-ANCA (anti-MPO)

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5
Q

Wegener’s Granulomatosis

A
  • positive ANCA
  • granulomas and patchy necrosis of a/v
  • necrosis above neck, lungs, and kidneys
  • episcleritis, saddle nose (necrosis of nasal septum)
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6
Q

ANCA responsible for Wegener’s Granulomatosis

A

c-ANCA (PR3-ANCA)

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7
Q

Kawasaki

A

damage to coronary arteries

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8
Q

Henoch-Schonlein

A

purpura and IgA nephropathy

- arthritis, GI bleeding

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9
Q

Cyroglobulinemia

A

marginally soluble protein precipitate in cool places or where blood is concentrated
(hep C Ab/Ag, rheumatoid factors)

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10
Q

Churg-Strauss

A

“allergic granulomatosis”

  • abundant eosinophils
  • caused by LT antagonist Rx for asthma
  • mononeuritis multiplex is common
  • occulsion of heart microvasculature causes death
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11
Q

Behcet’s Disease

A

AI against HSP

  • mouth lesions(HLA-B51)
  • eye lesions (hypopyon in lower eyelid)
  • genital, skin lesions
  • pathergy test positive
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12
Q

What is the pathergy test for?

A

Behcet’s

- ulcer will form at site of sterile needle prick

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13
Q

What is amyloid?

A

proteins that accumulate in beta-pleated form

  • effects many organs
  • can lead to carpal tunnel
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14
Q

How does amyloid stain?

A

congo red with apple green birefringence

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15
Q

Amyloid AA

A

serum amyloid associated protein

- prolonged inflammation w/ macrophages, osteomyelitis, TB, leprosy, rheumatoid arthritis

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16
Q

Amyloid AL

A

immunoglobulin light chains

- plasma cell neoplasia

17
Q

Amyloid ATTR (AF)

A

transthyretin

  • may get liver transplant
  • carries vitamin A
18
Q

Amyloid b2m (AH)

A

beta-two microglobulin

  • HLA light chains
  • long standing kidney failure
19
Q

What amyloid causes restrictive cardiomyopathy (stiff heart)?

A

Amyloid AL

20
Q

What do patients with defective innate immunity have difficulty with?

A

bacteria

21
Q

Chediak-Higashi disease

A

defect in phagolysosome function

  • also interferes with melanosome formation
  • causes neuropathy
22
Q

What is the defect mechanism in Chronic Granulomatous Diseases?

A

oxygen-dependent bacterial killing

- require macrophage system to handle bacteria

23
Q

What do patients with complement component deficiencies have trouble fighting?

A

Bacteria

24
Q

What disease do C2/C4/C1q deficiencies mimic?

A

Lupus

- alternate pathway works to handle bacteria

25
Q

What problems come from a C3 deficiency?

A

bacteria and with immune complex membranoproliferative glomerulonephritis

26
Q

What problems come from a C5-C9 deficiency?

A

Difficulty with bacteria

- most importantly neisseria

27
Q

What defect causes hereditary angioedema?

A

C1-esterase inhibitor

28
Q

Deficiency in T-cells can lead to increased risk for –

A

candida, CMV, pneumocystis

29
Q

Deficiency in B-cells can lead to increased risk for –

A

bacterial infections (once mom’s Ab clear)

30
Q

Severe Combined Immunodeficiency

A

Thymus produces few T-cells

- X-linked is most common (mutation of IL2RG – g-chain of IL)

31
Q

Bruton’s disease

A

X-linked agammaglobulinemia

  • lack of bruton’s tyrosine kinsase (B-cells do not mature)
  • trouble with bacteria
32
Q

DiGeorge Syndrome

A

“thymic dysembryogenesis”

  • lack parathyroids, thymus
  • have midline cardiac defects
33
Q

Hyper-IgM

A

patients can’t make IgG, IgA, IgE

  • mutation of CD40 on X-Chr
  • low neutrophils/platelets
  • pneumocystis infections
34
Q

Isolated IgA deficiency

A

Anaphylaxis occurs from donor IgA

- seen at second blood transfusion

35
Q

Sex-Linked Lymphoproliferative syndrome

A

Mutant SH2D1A – signal transduction protein

  • poor response to viruses allows Epstein-Barr virus to produce over-growth of lymphocytes
  • destroy liver/marrow
36
Q

Wiskott-Aldrich

A

X-linked, tiny platelets, eczema, deficient T-cells, WASp locus, ataxia, telangiectasia

37
Q

Hyper-IgE

A

“Job’s syndrome”

  • too much IgE, faulty neutrophil movement
  • Staph causes problems

MOD (7 decks)

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